VIPoma

VIPomas are rare neuroendocrine tumours (NETs). They start in the neuroendocrine cells that make the hormone VIP.

VIP stands for vasoactive intestinal peptide. VIP relaxes the muscles in the stomach and bowel. And it helps to control the balance of sugar, salt and water in the gastrointestinal (GI) tract.

Doctors sometimes group NETs depending on whether they make and release abnormal levels of hormones:

• Non-functioning NETs make and release normal levels of hormones.
• Functioning NETs make and release abnormal levels of hormones. 

VIPomas are a type of functioning NET.

They are also called gastro entero pancreatic neuroendocrine tumours (GEP NETs). Or pancreatic neuroendocrine tumours (pNETs).

The pancreas

The pancreas is part of our digestive system .

The pancreas is quite high up in the tummy (abdomen). It lies across your body where the ribs meet at the bottom of the breastbone, just behind your stomach. It is about 6 inches long (15 centimetres) and shaped like a leaf. 

The pancreas has 3 parts:

• the wide part is the head

• the thin end is the tail

• the part in the middle is the body

The pancreatic duct is a tube that collects the digestive juices made by the pancreas. It carries them into the first part of the bowel (duodenum).

Where does VIPoma start?

Around 90 out of every 100 VIPomas (around 90%) start in the pancreas. Most of these start in the tail of the pancreas.

More rarely, VIPomas can start in the:

• lungs
• bowel
• liver
• adrenal glands

VIPomas often grow slowly and are diagnosed early. But some people are diagnosed when their cancer has already spread to other parts of the body. 

The most common places where VIPomas spread to are the liver and lymph nodes .

How common is VIPoma?

VIPomas are very rare. Less than 1 person in every million develop a VIPoma every year.

Symptoms of VIPoma

VIPomas make large amounts of the hormone VIP. VIP relaxes the muscles in the stomach and bowel. It also helps to control the balance of sugar, salt and water in the gastrointestinal (GI) tract.

Symptoms usually develop slowly. In most people, the tumour has already started to spread to other parts of the body by the time it is diagnosed.

You usually have symptoms caused by the increase in the amount of VIP in your body. Symptoms include loose or watery poo (diarrhoea) which can be severe. 

Other symptoms might include:

• dehydration
• a low level of potassium in your blood
• weight loss
• flushing of the skin of your face, neck and chest
• tummy pain

The diarrhoea can be severe. You might have large amounts of watery poo (stools). Some people need to go to the toilet more than 20 times a day. You can have diarrhoea even when you haven’t eaten beforehand.

Dehydration and low potassium can cause symptoms such as:

• feeling thirsty
• feeling weak and tired
• headaches and feeling dizzy
• weak muscles and muscle cramps

Verner Morrison syndrome

Verner Morrison syndrome is the name given to the collection of symptoms caused by having high levels of the hormone VIP. It’s also called:

• VIPoma syndrome
• pancreatic cholera
• watery diarrhoea, hypokalaemia and hypochlorhydria or achlorhydria syndrome (WDHA)

Seeing your GP and referral to a specialist

If you have symptoms, you usually see your GP first. They might organise some tests. Your GP will decide if you need to see a specialist. They might refer you to a gastroenterology specialist. These doctors specialise in problems affecting the digestive system. 

VIPomas are rare tumours. So your doctor might ask you to have tests that check for other conditions first.

If your doctor suspects cancer, you have tests to check the type of cancer you have. If tests show that you have a VIPoma, your specialist will refer you to a team of doctors and specialist nurses who have expertise in treating NETs

Tests for VIPoma

Tests are to check the type of cancer you have. Tests also show the size of the cancer and whether it has spread. This helps your doctor plan your treatment.

Tests might include:

• checking your blood VIP level - you mustn’t eat anything before this test 
• blood tests to check your general health and to check the levels of other substances raised by NETs
• a blood test to check for a rare inherited condition called multiple endocrine neoplasia 1 (MEN1)
• a CT scan
• an MRI scan
• radioactive scans – these are octreotide scans (or octreoscans ) and gallium PET scans
• an endoscopic ultrasound scan of your tummy to look at your stomach, pancreas and bile ducts , and to take biopsies

You may have other tests depending on your symptoms. Your doctor will tell you which tests you need.

Grades and stages of VIPoma

Grade

The pathologist  looks at a sample of VIPoma cells under a microscope. They look at:

• how abnormal the cancer cells look – doctors call this differentiation
• how quickly or slowly they are dividing and growing – this is grading

VIPomas are well differentiated cancers. This means the NET cells look abnormal. But they still have some similarities to normal neuroendocrine cells.

Stage

You have tests to find out the size of the VIPoma and whether it has spread. This is your stage. Doctors use your stage to plan your treatment.

There are different ways to stage pancreatic NETs. Doctors usually use the TNM system or number stages. There are 4 main stages of pancreatic NETs - stage 1 to stage 4.

Your doctor will use a different staging system if your VIPoma starts outside the pancreas. They can tell you more about that.

Risks and causes

A risk factor is anything that increases your risk of getting a disease. 

We don’t know what causes most neuroendocrine cancers. But there are some risks factors that can increase your risk of developing it. Having one of these risk factors doesn’t mean that you will definitely develop a cancer. 

The risk factors for pancreatic NETs include:

• an inherited condition called multiple endocrine neoplasia 1 (MEN1)  
• having a family history of cancer 

Treatment for VIPoma

The first treatment you have is to control your symptoms. And to replace the fluids and minerals you may have lost because of diarrhoea. You then might have surgery to try to get rid of the cancer.

But surgery isn’t always possible. Some VIPomas may have already started to spread when you are diagnosed. Or you may not be well enough to have it. You continue to have treatment to help your symptoms if surgery isn’t an option.

Deciding which treatment you need

A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT).

The treatment you have depends on:

• where the NET is and its size
• how many tumours you have
• whether it has spread to other parts of the body
• your general health and fitness
• the symptoms you have

Your doctor will discuss your treatment. And tell you about its benefits and the possible side effects.

You are likely to have a clinical nurse specialist (CNS). They go to the MDT meetings. They can help answer your questions and support you. They are often your main point of contact throughout your treatment.

Types of treatment

Treatments to control symptoms at diagnosis

You usually have a drip into your bloodstream. This is to replace the fluid, potassium and other minerals you might have lost because of diarrhoea.

Exactly what you need depends on the potassium and mineral levels in your blood. And how dehydrated you are.

Somatostatin analogues

Somatostatin is a protein made naturally in the body. It does several things including slowing down the production of hormones. Somatostatin analogues are man made versions of somatostatin. 

You may have somatostatin analogues to try to slow down the tumour and help stop your diarrhoea. The most common drugs used are:

• octreotide (Sandostatin)
• lanreotide (Somatuline)

Support with weight loss and diet

You might see a dietitian if you have lost a lot of weight. They will give you advice about what you are eating and drinking. You might need to have:

• vitamin or mineral supplements
• drip or tube feeding if you have lost a lot of weight

Surgery

Surgery to remove the VIPoma is usually the main treatment. The type of surgery you have depends on whether the VIPoma started in your pancreas. Or if it started somewhere else in your body.

The information on this page is about surgery for VIPoma in the pancreas. For other types of VIPoma surgery, you can look at the surgery pages for cancer affecting the relevant part of the body.

Some of these are major operations and there are risks. But if the aim is to try to cure the NET, you might feel it is worth some risks. Talk to your doctor about the risks and benefits of your surgery.

There are different types of surgery. The type of operation you have depends on several factors. These include:

• where the cancer is in your pancreas
• the size of your cancer

You usually have open surgery. This means your surgeon makes a large cut in your tummy (abdomen) to remove the tumour. During the operation, they might also remove the nearby lymph nodes if they think your tumour is malignant (cancer).

Types of surgery to remove cancer from the pancreas

There are different operations:

Enucleation means the surgeon removes just the tumour. They leave the healthy pancreatic tissue behind. 

A distal pancreatectomy means the surgeon removes:

• the narrowest part of the pancreas
• the body of the pancreas

A pylorus preserving pancreaticoduodenectomy (PPPD) means the surgeon removes:

• the widest part of the pancreas
• the duodenum
• the gallbladder
• part of the bile duct

A Whipple’s operation means the surgeon removes:

• the widest part of the pancreas
• duodenum
• gallbladder
• part of the bile duct
• part of the stomach

If the cancer has spread to the liver, your surgeon might remove the liver tumours at the same time as the main surgery. Your surgeon may remove just the tumour or part of the liver.

Surgery to remove part of the tumour

Removing part of the tumour can reduce your symptoms. Your doctor will only suggest surgery if they think it’s possible to remove most of the tumour (at least 90%). They also call this debulking surgery.

Other treatments for VIPoma

You might have other treatments if:

• you can’t have surgery to remove the NET
• the NET has spread to another part of the body
• the NET came back after the initial treatment

These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options:

• somatostatin analogues - drugs include octreotide and lanreotide
• peptide receptor radionuclide therapy (PRRT) - this uses a radioactive medicine to treat the NET
• chemotherapy – drugs include temozolomide, fluorouracil, capecitabine, streptozotocin
• targeted cancer drugs – drugs include everolimus and sunitinib

For cancer that has spread to your liver you might have:

• trans arterial embolisation (TAE) to cancer in your liver
• radiofrequency ablation
• surgery to remove cancer from your liver
• a liver transplant – this is a rare treatment for NETs

Research and clinical trials

Doctors are always trying to improve treatments and reduce the side effects. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or to look at different combinations of existing treatments.

Follow up

You usually have follow up appointments after treatment. This is to check how you are and see whether you have any problems or worries. 

You might also have tests on some visits. The tests might include:

• blood tests
• an MRI or CT scan

How often you see your doctor depends on:

• the grade of your NET
• whether it has spread to other parts of the body
• what treatment you have had
• any side effects you might have

It is important you contact your doctors if you have any worrying symptoms between appointments.

Survival (prognosis) for VIPoma

The best person to talk to you about your outlook (prognosis ) is your doctor. Not everyone wants to know. People cope differently with cancer and want different information.

Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.

Your prognosis will depend on the type of NET you have and what stage it is.

Living with VIPoma

Diarrhoea can have a massive impact on your life and how you are feeling. We have some information about diarrhoea and diet.

You might have physical changes to your body because of your cancer or the treatment. These changes can be hard to cope with and can affect the way you feel about yourself. It can help to talk to friends and family. Or join a support group to meet people in a similar situation.

We have a discussion forum called Cancer Chat. It is a place for anyone affected by cancer. You can share experiences, stories and information with other people with cancer.

You might need practical advice about benefits or financial help. There is help and support available. There are also organisations to support and provide information to people affected by neuroendocrine cancer.